肾小管酸中毒195例临床诊疗分析

发布时间：2018-10-16 16:25

【摘要】：目的通过对大样本肾小管酸中毒(RTA)病例进行分析,总结该疾病的病因、临床特点、误(漏)诊原因及有关的治疗方法,以提高早期诊断率、降低误诊误治率。方法收集中国人民解放军总医院近20年收治的195例肾小管酸中毒的患者的所有的临床资料,回顾性地分析肾小管酸中毒患者的临床及实验室检查的相关资料,以及错误诊断或者遗漏诊断的情况、治疗转归的情况以及并发症的情况等。结果本组病例中,男性患者51例,女性患者144例,年龄2-79岁。(1)病因:RTA的病因多种多样,涵盖Sjogren综合征、髓质海绵肾、肾毒性药物、慢性乙型肝炎等等。原发性RTA占23.1% ;继发性RTA占76.9%。病因涵盖干燥综合征(42. 6%)、慢性肾脏疾病(12.8 %)、肾毒性药物(4. 1% )等。其中Sjogren综合征为最主要的肾小管酸中毒的继发性的病因。(2)临床表现:肾小管酸中毒的患者的临床表现比较复杂多样,常涉及多个系统和器官,比较容易导致多种并发症的发生,而患者的临床表现却缺乏特异性。首发症状中乏力、萎靡69. 7% ,烦渴、多饮、多尿45.6%,肢瘫39.5% ,骨关节痛33.8%等。患者常常分散就诊于多个科室,初次诊断时错误诊断和遗漏诊断的几率比较大(21%)。 (3)诊断治疗:对RTA患者进行明确的病因及表型甄别并给予相应的对症治疗及对因治疗,特别是对于活动期干燥综合征患者应用免疫抑制治疗,大部分患者治疗效果明显。在一般的纠正酸中毒、水和电解质水平紊乱的基础上,对于原发病为活动期干燥综合征(83例)、SLE(2例)、RA(2例)应用糖皮质激素(GCS或Glucocorticoid)口服或联合应用环磷酰胺(CTX或Cyclophosphamide)静脉滴注治疗,获得病情长期缓解。结论对RTA患者进行明确的病因及表型甄别并给予相应的对症治疗,特别是对于活动期自身免疫性疾病患者应用免疫抑制治疗,能够改善患者长期预后。临床医师应当加强对RTA这种病因复杂、临床表现多变的代谢性疾病的认识,并能够做出早期明确诊断和正确施治,从而改善患者生活质量、降低远期并发症发生。
[Abstract]:Objective to summarize the etiology, clinical characteristics, causes of misdiagnosis and treatment of renal tubular acidosis (RTA) in a large sample, so as to improve the early diagnosis rate and reduce the misdiagnosis and mistreatment rate. Methods all the clinical data of 195 patients with renal tubular acidosis admitted to the General Hospital of the Chinese people's Liberation Army in recent 20 years were collected, and the clinical and laboratory data of the patients with renal tubular acidosis were analyzed retrospectively. And false diagnosis or omission of the diagnosis, the outcome of treatment and complications, and so on. Results there were 51 male and 144 female patients aged 2-79 years. (1) the etiology of RTA was varied, including Sjogren syndrome, medullary sponge kidney, nephrotoxic drugs, chronic hepatitis B and so on. Primary RTA was 23. 1%, secondary RTA was 76. 9%. The etiology covers Sjogren's syndrome (42. 7%). Chronic kidney disease (12.8%), nephrotoxic drugs (4. 8%). 1%) etc. Sjogren syndrome is the main secondary cause of renal tubular acidosis. (2) Clinical manifestations: the clinical manifestations of patients with renal tubular acidosis are complex and diverse, often involving multiple systems and organs. But the patient's clinical manifestation lacks specificity. Initial symptoms of fatigue, malaise 69. 7%, thirsty, drink, polyuria 45.6%, limb paralysis 39.5%, bone and joint pain 33.8%. Patients are often scattered in multiple departments, the first diagnosis of false diagnosis and missed diagnosis is relatively high (21%). (3) diagnosis and treatment: the etiology and phenotype of patients with RTA were identified, and the corresponding symptomatic treatment and therapy were given, especially for active Sjogren's syndrome patients with immunosuppressive therapy. On the basis of general correction of acidosis and disturbance of water and electrolyte levels, active Sjogren's syndrome (83 cases of), SLE (2 cases of), RA (2 cases) was treated with glucocorticoid (GCS or Glucocorticoid) orally or combined with cyclophosphamide (CTX or Cyclophosphamide) intravenous drip. Get a long remission. Conclusion Immunosuppressive therapy can improve the long-term prognosis of patients with RTA by identifying their etiology and phenotypes and giving corresponding symptomatic treatment, especially for active autoimmune diseases. Clinicians should strengthen the understanding of metabolic disease with complex etiology and changeable clinical manifestations and make early diagnosis and correct treatment so as to improve the quality of life of patients and reduce the occurrence of long-term complications.
【学位授予单位】：中国人民解放军医学院
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R692.6

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